wobbler

Summary

Gene Symbol: wobbler
Description: VPS54 GARP complex subunit
Alias: Hcc8, Vps54l, mSLP8, vacuolar protein sorting-associated protein 54, tumor antigen SLP-8p homolog, vacuolar protein sorting 54
Species: mouse
Products:     wobbler

Top Publications

  1. Dennis J, Citron B. Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein. Neuroscience. 2009;158:745-50 pubmed publisher
    b>Wobbler mice model motor neuron disease with a substantial decline in motor neurons. TDP-43 is a nucleic acid binding protein that accumulates, along with ubiquitin, in the cytoplasm of amyotrophic lateral sclerosis (ALS) motor neurons...
  2. Wedemeyer N, Lengeling A, Ronsiek M, Korthaus D, Baer K, Wuttke M, et al. YAC contigs of the Rab1 and wobbler (wr) spinal muscular atrophy gene region on proximal mouse chromosome 11 and of the homologous region on human chromosome 2p. Genomics. 1996;32:447-54 pubmed
    ..g., proximal mouse chromosome 11 (Chr 11) and human chromosome 2p (Chr 2p). We have localized the wobbler spinal atrophy gene wr to proximal mouse Chr 11, tightly linked to Rab1, a gene coding for a small GTP-binding ..
  3. Gonzalez Deniselle M, Carreras M, Garay L, Gargiulo Monachelli G, Meyer M, Poderoso J, et al. Progesterone prevents mitochondrial dysfunction in the spinal cord of wobbler mice. J Neurochem. 2012;122:185-95 pubmed publisher
    In the Wobbler mouse, a mutation of the Vps54 protein increases oxidative stress in spinal motoneurons, associated to toxic levels of nitric oxide and hyperactivity of nitric oxide synthase (NOS)...
  4. Tomiyama M, Kannari K, Nunomura J, Oyama Y, Takebe K, Matsunaga M. Quantitative autoradiographic distribution of glutamate receptors in the cervical segment of the spinal cord of the wobbler mouse. Brain Res. 1994;650:353-7 pubmed
    The reduction of glutamate content has been observed in the spinal cord of the wobbler mouse, a purported model of amyotrophic lateral sclerosis (ALS)...
  5. Ikeda K, Iwasaki Y, Kinoshita M, Marubuchi S, Ono S. T-588, a novel neuroprotective agent, delays progression of neuromuscular dysfunction in wobbler mouse motoneuron disease. Brain Res. 2000;858:84-91 pubmed
    ..We attempted to determine whether T-588 treatment ameliorates neuromuscular dysfunction in the wobbler mouse, an animal model of motoneuron disease (MND)...
  6. Gonzalez Deniselle M, Garay L, Gonzalez S, Guennoun R, Schumacher M, De Nicola A. Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease. Exp Neurol. 2005;195:518-23 pubmed
    The Wobbler mouse, a mutant characterized by motoneuron degeneration in the cervical spinal cord, has been used to test the efficacy of novel treatments for human motoneuron diseases (HMD)...
  7. Boillee S, Peschanski M, Junier M. The wobbler mouse: a neurodegeneration jigsaw puzzle. Mol Neurobiol. 2003;28:65-106 pubmed
    ..This can be exemplified by the work performed on the wobbler mouse, one of the oldest and most extensively studied models, which is reviewed here.
  8. Mitsumoto H, Kurahashi K, Jacob J, McQuarrie I. Retardation of fast axonal transport in wobbler mice. Muscle Nerve. 1993;16:542-7 pubmed
    ..examined fast and slow components of anterograde axonal transport in the less-affected hindlimb motor neurons of wobbler mice...
  9. Nieto Gonzalez J, Moser J, Lauritzen M, Schmitt John T, Jensen K. Reduced GABAergic inhibition explains cortical hyperexcitability in the wobbler mouse model of ALS. Cereb Cortex. 2011;21:625-35 pubmed publisher
    ..Here, we studied the GABAergic system in cortex using patch-clamp recordings in the wobbler mouse, a model of ALS...

More Information

Publications75

  1. Santoro B, Bigini P, Levandis G, Nobile V, Biggiogera M, Botti F, et al. Evidence for chronic mitochondrial impairment in the cervical spinal cord of a murine model of motor neuron disease. Neurobiol Dis. 2004;17:349-57 pubmed
    Profound alteration of the oxygen consumption rate (QO2) is present in the cervical spinal cord (CS) of the wobbler mice aged 12 weeks (wr12)...
  2. Rathke Hartlieb S, Schmidt V, Jockusch H, Schmitt John T, Bartsch J. Spatiotemporal progression of neurodegeneration and glia activation in the wobbler neuropathy of the mouse. Neuroreport. 1999;10:3411-6 pubmed
    The wobbler mouse (phenotype WR; genotype wr/wr) has been investigated as a model for neurodegenerative diseases like SMA and ALS...
  3. Pioro E, Wang Y, Moore J, Ng T, Trapp B, Klinkosz B, et al. Neuronal pathology in the wobbler mouse brain revealed by in vivo proton magnetic resonance spectroscopy and immunocytochemistry. Neuroreport. 1998;9:3041-6 pubmed
    ..to measure the in vivo signal of N-acetylaspartate (NAA), a putative neuronal marker, in the brain of the mutant wobbler mouse, a model of motor neuron disease...
  4. Pernas Alonso R, Schaffner A, Perrone Capano C, Orlando A, Morelli F, Hansen C, et al. Early upregulation of medium neurofilament gene expression in developing spinal cord of the wobbler mouse mutant. Brain Res Mol Brain Res. 1996;38:267-75 pubmed
    Homozygous wobbler mouse mutants develop a progressive paralysis due to spinal motoneuron degeneration...
  5. Ikeda K, Iwasaki Y, Tagaya N, Shiojima T, Kinoshita M. Neuroprotective effect of cholinergic differentiation factor/leukemia inhibitory factor on wobbler murine motor neuron disease. Muscle Nerve. 1995;18:1344-7 pubmed
  6. Blondet B, Hantaz Ambroise D, Ait Ikhlef A, Cambier D, Murawsky M, Rieger F. Astrocytosis in wobbler mouse spinal cord involves a population of astrocytes which is glutamine synthetase-negative. Neurosci Lett. 1995;183:179-82 pubmed
    Mice affected by the wobbler mutation are characterized by a muscular atrophy associated with motoneuron degeneration...
  7. Pollin M, McHanwell S, Slater C. Loss of motor neurons from the median nerve motor nucleus of the mutant mouse 'wobbler'. J Neurocytol. 1990;19:29-38 pubmed
    This paper describes the location and number of motor neurons in the median nerve pool of wobbler mice and normal littermates as determined by retrograde labelling of the cut median nerve with horseradish peroxidase (HRP) in animals from ..
  8. Augustin M, Heimann P, Rathke S, Jockusch H. Spinal muscular atrophy gene wobbler of the mouse: evidence from chimeric spinal cord and testis for cell-autonomous function. Dev Dyn. 1997;209:286-95 pubmed
    ..In this species, a number of mutations impair spermiogenesis in addition to neuron survival. Among these, the wobbler mutation on proximal chromosome 11 of the mouse leads to motoneuron degeneration in brain stem and spinal cord ..
  9. Rathke Hartlieb S, Budde P, Ewert S, Schlomann U, Staege M, Jockusch H, et al. Elevated expression of membrane type 1 metalloproteinase (MT1-MMP) in reactive astrocytes following neurodegeneration in mouse central nervous system. FEBS Lett. 2000;481:227-34 pubmed
    ..matrix metalloproteinase 1 (MT1-MMP) and tissue inhibitors 1-4 of MMPs (TIMPs) in brain stem and spinal cord of wobbler (WR) mutant mice affected by progressive neurodegeneration and astrogliosis...
  10. Blondet B, Carpentier G, Aït Ikhlef A, Murawsky M, Rieger F. Motoneuron morphological alterations before and after the onset of the disease in the wobbler mouse. Brain Res. 2002;930:53-7 pubmed
    The wobbler mutant mouse displays a recessively inherited neurological disease with degeneration of motoneurons and is considered to be an animal model for human motoneuron diseases...
  11. Bartsch J, Wildeboer D, Koller G, Naus S, Rittger A, Moss M, et al. Tumor necrosis factor-alpha (TNF-alpha) regulates shedding of TNF-alpha receptor 1 by the metalloprotease-disintegrin ADAM8: evidence for a protease-regulated feedback loop in neuroprotection. J Neurosci. 2010;30:12210-8 pubmed publisher
    ..In motor neuron (MN) disease of the mouse, exemplified by the model wobbler (WR), TNF-alpha causes upregulation of the metalloprotease-disintegrin ADAM8 (A8) in affected brain regions, ..
  12. Mitsumoto H, Gambetti P. Impaired slow axonal transport in wobbler mouse motor neuron disease. Ann Neurol. 1986;19:36-43 pubmed
    We studied slow axonal transport and morphometry of forelimb axons in wobbler mice and controls. In wobbler mice, the total radioactivity migrating with the slow transport was decreased by 50%...
  13. Ikeda K, Iwasaki Y, Kinoshita M. Neuronal nitric oxide synthase inhibitor, 7-nitroindazole, delays motor dysfunction and spinal motoneuron degeneration in the wobbler mouse. J Neurol Sci. 1998;160:9-15 pubmed
    ..There are increased amounts of nitric oxide and SOD hypoactivities in the brain and spinal cord of wobbler mice. NOS is also induced in the vacuolated spinal motoneurons or axons in this animal...
  14. Ikeda K, Kinoshita M, Iwasaki Y, Tagaya N, Shiojima T. Lecithinized superoxide dismutase retards wobbler mouse motoneuron disease. Neuromuscul Disord. 1995;5:383-90 pubmed
    ..We have shown that SOD treatment improves neuromuscular dysfunction and morphological changes in wobbler mouse motoneuron disease...
  15. Xu G, Dave K, Moraes C, Busto R, Sick T, Bradley W, et al. Dysfunctional mitochondrial respiration in the wobbler mouse brain. Neurosci Lett. 2001;300:141-4 pubmed
    ..In the present study we tested the hypothesis that alterations in mitochondrial physiology occur in the brain of wobbler mice...
  16. Heimann P, Laage S, Jockusch H. Defect of sperm assembly in a neurological mutant of the mouse, wobbler (WR). Differentiation. 1991;47:77-83 pubmed
    In the wobbler (WR) mouse, a neuromuscular mutant characterized by a motoneuron degeneration and male infertility, the cellular basis of the defect in spermiogenesis was studied by light and electron microscopy as well as by lectin ..
  17. Krieger C, Perry T, Hansen S, Mitsumoto H, Honore T. Excitatory amino acid receptor antagonist in murine motoneuron disease (the wobbler mouse). Can J Neurol Sci. 1992;19:462-5 pubmed
    ..The wobbler mouse is an autosomal recessive mutant which develops progressive neurological symptoms secondary to motoneuron ..
  18. Karlsson P, Droce A, Moser J, Cuhlmann S, Padilla C, Heimann P, et al. Loss of vps54 function leads to vesicle traffic impairment, protein mis-sorting and embryonic lethality. Int J Mol Sci. 2013;14:10908-25 pubmed publisher
    The identification of the mutation causing the phenotype of the amyotrophic lateral sclerosis (ALS) model mouse, wobbler, has linked motor neuron degeneration with retrograde vesicle traffic...
  19. Pernas Alonso R, Perrone Capano C, Volpicelli F, di Porzio U. Regionalized neurofilament accumulation and motoneuron degeneration are linked phenotypes in wobbler neuromuscular disease. Neurobiol Dis. 2001;8:581-9 pubmed
    ..Increased expression of medium neurofilament (NFM) is an early molecular marker of wobbler mouse, an animal model of motoneuron disease...
  20. Resch K, Korthaus D, Wedemeyer N, Lengeling A, Ronsiek M, Thiel C, et al. Homology between human chromosome 2p13.3 and the wobbler critical region on mouse chromosome 11: comparative high-resolution mapping of STS and EST loci on YAC/BAC contigs. Mamm Genome. 1998;9:893-8 pubmed
    ..3 and mouse Chr 11, respectively, as well as the wobbler (wr) critical region of the mouse...
  21. Diana V, Ottolina A, Botti F, Fumagalli E, Calcagno E, De Paola M, et al. Neural precursor-derived astrocytes of wobbler mice induce apoptotic death of motor neurons through reduced glutamate uptake. Exp Neurol. 2010;225:163-72 pubmed publisher
    ..astrocytes derived from adult neural precursor cells (NPCs) obtained from the subventricular zone (SVZ) of wobbler mice display metabolic traits of the wobbler astrocytes in situ and in primary culture...
  22. Bigini P, Bastone A, Mennini T. Glutamate transporters in the spinal cord of the wobbler mouse. Neuroreport. 2001;12:1815-20 pubmed
    We studied the role of glutamate excitotoxicity in motor neuron degeneration in the wobbler mouse (wr/wr), a model of amyotrophic lateral sclerosis and spinal muscular atrophies...
  23. Murakami T, Mastaglia F, Bradley W. Reduced protein synthesis in spinal anterior horn neurons in wobbler mouse mutant. Exp Neurol. 1980;67:423-32 pubmed
  24. Ikeda K, Kinoshita M, Tagaya N, Shiojima T, Taga T, Yasukawa K, et al. Coadministration of interleukin-6 (IL-6) and soluble IL-6 receptor delays progression of wobbler mouse motor neuron disease. Brain Res. 1996;726:91-7 pubmed
    ..Although CNTF or LIF treatment attenuates motor deficits in wobbler mouse motor neuron disease (MND), neuroprotective effects of IL-6 on this animal have not yet been clarified...
  25. Pérez Victoria F, Abascal Palacios G, Tascón I, Kajava A, Magadán J, Pioro E, et al. Structural basis for the wobbler mouse neurodegenerative disorder caused by mutation in the Vps54 subunit of the GARP complex. Proc Natl Acad Sci U S A. 2010;107:12860-5 pubmed publisher
    ..of leucine-967 to glutamine in the Vps54 subunit of GARP is responsible for spinal muscular atrophy in the wobbler mouse, an animal model of amyotrophic lateral sclerosis. The crystal structure at 1...
  26. Staunton L, Jockusch H, Ohlendieck K. Proteomic analysis of muscle affected by motor neuron degeneration: the wobbler mouse model of amyotrophic lateral sclerosis. Biochem Biophys Res Commun. 2011;406:595-600 pubmed publisher
    ..The wobbler mouse (phenotype WR, genotype wr/wr) is an established animal model of human motor neuron disease and is ..
  27. Kaupmann K, Simon Chazottes D, Guenet J, Jockusch H. Wobbler, a mutation affecting motoneuron survival and gonadal functions in the mouse, maps to proximal chromosome 11. Genomics. 1992;13:39-43 pubmed
    The wobbler mouse (genotype wr/wr) has been considered as an animal model for human neurodegenerative disorders. In the homozygous condition, the autosomal mutation wobbler (wr) causes a motoneuron disease and gonadal dysfunction...
  28. Moser J, Bigini P, Schmitt John T. The wobbler mouse, an ALS animal model. Mol Genet Genomics. 2013;288:207-29 pubmed publisher
    This review article is focused on the research progress made utilizing the wobbler mouse as animal model for human motor neuron diseases, especially the amyotrophic lateral sclerosis (ALS)...
  29. Fuchs S, Resch K, Thiel C, Ulbrich M, Platzer M, Jockusch H, et al. Comparative transcription map of the wobbler critical region on mouse chromosome 11 and the homologous region on human chromosome 2p13-14. BMC Genet. 2002;3:14 pubmed
    To support the positional cloning of the mouse mutation wobbler (wr) the corresponding regions on human Chr2p13-14 and mouse Chr11 were analyzed in detail and compared with respect to gene content, order, and orientation...
  30. Bose P, Fielding R, Vacca Galloway L. Effects of assisted feeding on Wobbler mouse motoneuron disease and on serotonergic and peptidergic sprouting in the cervical spinal ventral horn. Brain Res Bull. 1999;48:429-39 pubmed
    The Wobbler mouse is used as a model of human motoneuron disease (MND)...
  31. Tsuzaka K, Ishiyama T, Pioro E, Mitsumoto H. Role of brain-derived neurotrophic factor in wobbler mouse motor neuron disease. Muscle Nerve. 2001;24:474-80 pubmed
    ..neuroprotective for motoneurons undergoing degeneration, including those in natural motor neuron disease (MND) in wobbler mice...
  32. Dave K, Raval A, Purroy J, Kirkinezos I, Moraes C, Bradley W, et al. Aberrant deltaPKC activation in the spinal cord of Wobbler mouse: a model of motor neuron disease. Neurobiol Dis. 2005;18:126-33 pubmed
    ..The goal of the present study was to define whether deltaPKC activation occurs in Wobbler mouse spinal cord (a model of motor neuron disease)...
  33. Fumagalli E, Bigini P, Barbera S, De Paola M, Mennini T. Riluzole, unlike the AMPA antagonist RPR119990, reduces motor impairment and partially prevents motoneuron death in the wobbler mouse, a model of neurodegenerative disease. Exp Neurol. 2006;198:114-28 pubmed
    The wobbler mouse is one of the most useful models of motoneuron degeneration, characterized by selective motoneuronal death in the cervical spinal cord...
  34. Mitsumoto H, Ikeda K, Klinkosz B, Cedarbaum J, Wong V, Lindsay R. Arrest of motor neuron disease in wobbler mice cotreated with CNTF and BDNF. Science. 1994;265:1107-10 pubmed
    ..of either factor alone has been shown to slow, but not to arrest, progression of motor neuron dysfunction in wobbler mice, an animal model of motor neuron disease...
  35. Dave K, Bradley W, Perez Pinzon M. Early mitochondrial dysfunction occurs in motor cortex and spinal cord at the onset of disease in the Wobbler mouse. Exp Neurol. 2003;182:412-20 pubmed
    The Wobbler mouse is recognized as an animal model for motoneuron disease that exhibits motoneuron pathology. We have recently demonstrated the occurrence of mitochondrial dysfunction in the Wobbler mouse brain...
  36. Gonzalez Deniselle M, Gonzalez S, Lima A, Wilkin G, De Nicola A. The 21-aminosteroid U-74389F attenuates hyperexpression of GAP-43 and NADPH-diaphorase in the spinal cord of wobbler mouse, a model for amyotrophic lateral sclerosis. Neurochem Res. 1999;24:1-8 pubmed
    The wobbler mouse suffers an autosomal recessive mutation producing severe neurodegeneration and astrogliosis in spinal cord. It has been considered a model for amyotrophic lateral sclerosis...
  37. Schmitt John T, Drepper C, Mussmann A, Hahn P, Kuhlmann M, Thiel C, et al. Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse. Nat Genet. 2005;37:1213-5 pubmed
    ..We identified the missense mutation L967Q in Vps54 in the wobbler mouse, an animal model of amyotrophic lateral sclerosis, and also characterized a lethal allele, Vps54(beta-geo)...
  38. Duchen L, Strich S. An hereditary motor neurone disease with progressive denervation of muscle in the mouse: the mutant 'wobbler'. J Neurol Neurosurg Psychiatry. 1968;31:535-42 pubmed
  39. Blondet B, Barlovatz Meimon G, Festoff B, Soria C, Soria J, Rieger F, et al. Plasminogen activators in the neuromuscular system of the wobbler mutant mouse. Brain Res. 1992;580:303-10 pubmed
    b>Wobbler, the neurological mutant mouse, carries an autosomal recessive gene (wr) and has been characterized as a model of lower motoneuron disorders with associated muscle atrophy, denervation and reinnervation...
  40. Meyer M, Gonzalez Deniselle M, Gargiulo Monachelli G, Garay L, Schumacher M, Guennoun R, et al. Progesterone effects on neuronal brain-derived neurotrophic factor and glial cells during progression of Wobbler mouse neurodegeneration. Neuroscience. 2012;201:267-79 pubmed publisher
    ..of brain-derived neurotrophic factor (BDNF) mRNA in the degenerating motoneurons from clinically afflicted Wobbler mice, whereas progesterone treatment reverts this depletion...
  41. Schindler C, Chen Y, Pu J, Guo X, Bonifacino J. EARP is a multisubunit tethering complex involved in endocytic recycling. Nat Cell Biol. 2015;17:639-50 pubmed publisher
    ..Depletion of syndetin or syntaxin 6 delays recycling of internalized transferrin to the cell surface. These findings implicate EARP in canonical membrane-fusion events in the process of endocytic recycling. ..
  42. Wei J, Zhang Y, Luo J, Wang J, Zhou Y, Miao H, et al. The GARP Complex Is Involved in Intracellular Cholesterol Transport via Targeting NPC2 to Lysosomes. Cell Rep. 2017;19:2823-2835 pubmed publisher
    ..We conclude that the GARP complex contributes to intracellular cholesterol transport by targeting NPC2 to lysosomes in a CI-MPR-dependent manner. ..
  43. Ulbrich M, Schmidt V, Ronsiek M, Mussmann A, Bartsch J, Augustin M, et al. Genetic modifiers that aggravate the neurological phenotype of the wobbler mouse. Neuroreport. 2002;13:535-9 pubmed
    The autosomal recessive mutation wobbler of the mouse (phenotype WR; genotype wr/wr) causes muscular atrophy due to motoneuron degeneration with 100% penetrance on the standard Mus musculus laboratorius C57BL/6J background...
  44. Gonzalez Deniselle M, Gonzalez S, Piroli G, Ferrini M, Lima A, De Nicola A. Glucocorticoid receptors and actions in the spinal cord of the Wobbler mouse, a model for neurodegenerative diseases. J Steroid Biochem Mol Biol. 1997;60:205-13 pubmed
    We have studied glucocorticoid receptors (GR) and actions in the spinal cord of the Wobbler mouse, a model for amyotrophic lateral sclerosis and infantile spinal muscular atrophy...
  45. Coulpier M, Junier M, Peschanski M, Dreyfus P. Bcl-2 sensitivity differentiates two pathways for motoneuronal death in the wobbler mutant mouse. J Neurosci. 1996;16:5897-904 pubmed
    ..We took advantage of the murine mutant wobbler, which undergoes progressive degeneration of the spinal and brainstem motoneurons...
  46. Pernas Alonso R, Schaffner A, Hansen C, Barker J, di Porzio U. Acetylcholine esterase and peripherin mRNA level decrease in wobbler mouse. Neuroreport. 1995;6:597-600 pubmed
    Homozygote wobbler mice develop motoneurone degeneration. Throughout development the expression of choline acetyltransferase, of trkC receptor and F3 adhesion molecule genes is similar in wobbler and wild-type spinal cord...
  47. Brooks B, Lust W, Andrews J, Engel W. Decreased spinal cord cGMP in murine (wobbler) spontaneous lower motor neuron degeneration. Arch Neurol. 1978;35:590-1 pubmed
    ..reduced by 80% in the cervical spinal cord and by 56% in the cerebellum of clinically affected homozygote "wobbler" mice compared to sex- and age-matched litter-mate clinically unaffected control mice...
  48. Jockusch H, Holland A, Staunton L, Schmitt John T, Heimann P, Dowling P, et al. Pathoproteomics of testicular tissue deficient in the GARP component VPS54: the wobbler mouse model of globozoospermia. Proteomics. 2014;14:839-52 pubmed publisher
    ..In the wobbler (WR) mouse, an L967Q missense mutation in the vesicular protein-sorting factor VPS54 causes motor neuron ..
  49. Chelmicka Schorr E, Sportiello M, Antel J, Arnason B. Acid protease activity in spinal cord and muscle in wobbler mouse. J Neurol Sci. 1982;56:141-5 pubmed
  50. Eve D, Dennis J, Citron B. Transcription factor p53 in degenerating spinal cords. Brain Res. 2007;1150:174-81 pubmed
    ..g., Bax) and inhibition of antiapoptotic genes (e.g., Bcl-2). A model of motor neuron loss is the wobbler mouse that exhibits rapid motor neuron cell death as well as motor deficit from 21 days after birth...
  51. Clowry G, McHanwell S. Brainstem motor nuclei respond differentially to degenerative disease in the mutant mouse wobbler. Neuropathol Appl Neurobiol. 2004;30:148-60 pubmed
    ..NADPH-d) histochemical reactivity, a marker for neurodegeneration, was studied in brainstem sections from mutant wobbler mice and their normal littermates during the motoneurone degeneration phase (3-8 weeks of age)...
  52. Bronstein J, Yamashita C, Farber D. Exclusion of the beta-subunit of type II calmodulin kinase for the wobbler spinal muscular atrophy gene. Brain Res Mol Brain Res. 1996;43:330-2 pubmed
    The wobbler mouse (wr) is an attractive model for studying motor neuron disease but the genetic defect is unknown...
  53. Ikeda K, Iwasaki Y, Tagaya N, Shiojima T, Kobayashi T, Kinoshita M. Neuroprotective effect of basic fibroblast growth factor on wobbler mouse motor neuron disease. Neurol Res. 1995;17:445-8 pubmed
    ..Here we report that it delays progression of motor neuron disease (MND) in the wobbler mouse...
  54. Yung K, Tang F, Vacca Galloway L. Decrease of enkephalins in cerebellum during Wobbler mouse motoneuron disease. Brain Res. 1992;599:175-80 pubmed
    The Wobbler mouse possesses an inherited motoneuron disease, which expresses itself primarily at cervical spinal levels and in cranial motor nuclei. Cell degeneration is sporatic and negligible in other motor regions of the brain (e.g...
  55. Yung K, Tang F, Fielding R, Du Y, Vacca Galloway L. Alteration in the levels of thyrotropin releasing hormone, substance P and enkephalins in the spinal cord, brainstem, hypothalamus and midbrain of the Wobbler mouse at different stages of the motoneuron disease. Neuroscience. 1992;50:209-22 pubmed
    ..methionine and leucine enkephalin) in the cervical spinal cord and other regions of the central nervous system of Wobbler mice by radioimmunoassays during several stages of the motoneuron disease compared with age- and sex-matched ..
  56. Bradley W, Jenkison M. Abnormalities of peripheral nerves in murine muscular dystrophy. J Neurol Sci. 1973;18:227-47 pubmed
  57. Jockusch H, Kaupmann K, Gronemeier M, Schleef M, Klocke R. Exploring the mammalian neuromuscular system by analysis of mutations: spinal muscular atrophy and myotonia. Prog Neurobiol. 1994;42:313-7 pubmed
    ..The wobbler mouse (phenotype WR, genotype wr/wr) suffers from muscular atrophy because of the degeneration of 20-40% of the ..
  58. Kaupmann K, Sendtner M, Stöckli K, Jockusch H. The Gene for Ciliary Neurotrophic Factor (CNTF) Maps to Murine Chromosome 19 and its Expression is Not Affected in the Hereditary Motoneuron Disease 'Wobbler' of the Mouse. Eur J Neurosci. 1991;3:1182-1186 pubmed
    ..The latter was carrying the gene wobbler (wr) for spinal muscular atrophy...
  59. Vacca Galloway L, Steinberger C. Substance P neurons sprout in the cervical spinal cord of the wobbler mouse: a model for motoneuron disease. J Neurosci Res. 1986;16:657-70 pubmed
    The mutant mouse, wobbler, possesses a recessively inherited degeneration of motoneurons and other ventral horn cells in the cervical spinal cord, and therefore it has been proposed as an animal model of human motoneuron disease...
  60. Korthaus D, Wedemeyer N, Wiegand C, Jockusch H. The gene for cytoplasmatic malate dehydrogenase, Mor2, is closely linked to the wobbler spinal muscular atrophy gene (wr). Mamm Genome. 1996;7:250 pubmed
  61. Andrews J. The fine structure of the cervical spinal cord, ventral root and brachial nerves in the wobbler (wr) mouse. J Neuropathol Exp Neurol. 1975;34:12-27 pubmed
    Much indirect evidence supports the prevailing concept that the wobbler mouse represents an inherited form of lower motor neuron degeneration, with resultant neurogenic muscular atrophy, due to an autosomal recessive gene (wr)...
  62. Ikeda K, Kinoshita M, Iwasaki Y. Basic fibroblast growth factor has neuroprotective effects on axotomy-induced spinal motoneuron death and wobbler mouse motoneuron disease. Muscle Nerve. 1996;19:794-5 pubmed
  63. Mitsumoto H, Ikeda K, Holmlund T, Greene T, Cedarbaum J, Wong V, et al. The effects of ciliary neurotrophic factor on motor dysfunction in wobbler mouse motor neuron disease. Ann Neurol. 1994;36:142-8 pubmed
    ..In the present study we tested the effects of recombinant rat or human ciliary neurotrophic factor in the wobbler mouse model of motor neuron disease...
  64. Thielsen K, Moser J, Schmitt John T, Jensen M, Jensen K, Holm M. The Wobbler mouse model of amyotrophic lateral sclerosis (ALS) displays hippocampal hyperexcitability, and reduced number of interneurons, but no presynaptic vesicle release impairments. PLoS ONE. 2013;8:e82767 pubmed publisher
    ..The wobbler mouse is a model of ALS, and like ALS patients the wobbler mouse displays cortical hyperexcitability...
  65. Meyer M, Gonzalez Deniselle M, Garay L, Sitruk Ware R, Guennoun R, Schumacher M, et al. The progesterone receptor agonist Nestorone holds back proinflammatory mediators and neuropathology in the wobbler mouse model of motoneuron degeneration. Neuroscience. 2015;308:51-63 pubmed publisher
    b>Wobbler mutant mice suffer from progressive motoneuron degeneration and glial cell reactivity in the spinal cord...
  66. Saberi D, Ott B, Dahlke C, Matschke V, Schmitt John T, Theiss C. The Spatiotemporal Pattern of Degeneration in the Cerebellum of the Wobbler Mouse. J Neuropathol Exp Neurol. 2016;75:347-57 pubmed publisher
    ..Degeneration in the spinal cord and motor cortex in the Wobbler mouse model of sporadic ALS have been documented, but alterations of the cerebellum during disease progression ..